Dyskeratosis congenita is an inherited disorder that usually presents in males, consisting of a triad of leukoplakia of the mucous membranes, nail dystrophy and skin pigmentation. Whilst most cases are X-linked, autosomal dominant and recessive forms have also been reported. The significance of the condition lies in premature mortality arising from either bone marrow failure or malignant change within the areas of mucosal leukoplakia. We present a case (X-linked recessive form) where the classic triad of signs were present, along with the development of lichenoid reaction in the buccal and labial mucosa. This is believed to be the first case in which such a reaction has been noted together with the oral hyperkeratosis. The identification of a white patch within the mouth of a child, in the absence of any other obvious cause must arouse suspicion of this rare condition. All clinicians should be aware of this rare genetic disorder so that early referral can be made and appropriate management instigated.